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Case 403


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32-year-old female patient with complaints of postprandial fullness, abdominal cramps and bulging of the abdomen that started 6 months ago. On physical examination, a palpable mass was observed in the left hypochondrium and flank. Fluid wave test was negative. Denies weight loss. Ultrasound (US) and computed tomography (CT) of the abdomen, colonoscopy and laparoscopy were requested, and the biopsy specimens revealed features of desmoplastic small round cell tumor.

Considering the clinical history, the imaging exams and the diagnosis indicated by the biopsy, which of the alternatives below describes the most appropriate medical approach?

a) Surgery

25%

b) Chemotherapy

25%

c) Radiation therapy

25%

d) Chemotherapy and surgery

25%
   

Image Analysis

Image 1 - Analysis: Computed tomography (CT) of the abdomen and pelvis, coronal reconstruction, after intravenous injection of iodinated contrast, portal phase. Presence of hypoattenuating masses with lobulated contours, multifocal distribution, density of soft tissue and irregular enhancement by the intravenous contrast agent, in the left hypochondriac and flank regions, measuring 11 cm in its largest diameter (delimited in red), next to the cecum (in blue) and in the pelvis (in green). Central areas with lack of contrast enhancement are noted, probably related to necrosis.

Image 2 - Analysis: CT of the abdomen and pelvis, coronal reconstruction, after intravenous injection of iodinated contrast, portal phase. Presence of multifocal masses and nodules with soft tissue density and irregular enhancement in left hypochondrium (delimited in yellow), left flank (in red), cecum (in blue), and pelvis (in green).

Image 3 - Analysis: Axial sections of CT of the abdomen and pelvis, after intravenous injection of iodinated contrast, portal phase. Multifocal masses are evident in the topography of the left hypochondrium and flank (A), the cecum, with a diameter of 3 cm (B), and the pouch of Douglas, measuring 8 cm in its largest axis (C). In C, notice the confluent lesions.

Image 4 - Analysis: Laparoscopy of the peritoneal cavity in the topography of the greater omentum (A and B), the pelvis (C) and the pouch of Douglas (D). Presence of multiple peritoneal implants (small arrows) and confluent tumor lesions (big arrows).

Image 5 - Analysis: Photo of hyperthermic intraperitoneal chemotherapy in the patient of the case. After cytoreduction, the heated chemotherapy is pumped into the abdominal cavity. Its local distribution in the abdomen allows its use in high concentrations, and hyperthermia increases the penetration into the peritoneal surface and the tumoricidal effect.

Image 6 – Analysis: Photo of the operative field with in situ view of the desmoplastic small round cell tumor and the complete cytoreduction surgical specimen, which involved parietal peritonectomy, omentectomy of greater omentum, total splenectomy, rectosigmoidectomy and pelvic peritonectomy with pan-hysterectomy.

Highlights

-       It is a rare and aggressive primary sarcoma of the peritoneum that is characterized by the translocation between chromosomes 11 and 22, and that affects young males in 90% of cases;

-       It is characterized by abdominal pain, ascites and weight loss, in addition to the presence of masses with soft tissue density in the peritoneal cavity at CT, without identification of the origin organ;

-       The diagnosis is made by histopathological and immunohistochemical exams, and usually occurs at an advanced stage, with sarcomatosis and metastases;

-       The standard treatment of desmoplastic small round cell tumor is based on a multimodal approach, which includes: neoadjuvant, intraoperative and adjuvant chemotherapy and cytoreductive surgery;

-       The prognosis of the disease is reserved even with aggressive treatment.

References

-       Hayes-Jordan A, LaQuaglia MP, Modak S. Management of desmoplastic small round cell tumor. Semin Pediatr Surg. 2016;25(5):299‐304. 

-       Subbiah, Vivek et al. Multimodality Treatment of Desmoplastic Small Round Cell Tumor: Chemotherapy and Complete Cytoreductive Surgery Improve Patient Survival. Clin Cancer Res. 2018;24(19):4865‐4873.

-       A. C. Morani, et al. Desmoplastic Small Round Cell Tumor: Imaging Pattern of Disease at Presentation. AJR Am J Roentgenol. 2019 Mar;212(3):W45-W54

-       Izquierdo, F.J et al. The Chicago Consensus on Peritoneal Surface Malignancies: Management of Desmoplastic Small Round Cell Tumor, Breast, and Gastrointestinal Stromal Tumors. Ann Surg Oncol 27, 1793–1797 (2020).

Author

Larissa Gonçalves Rezende, 4th year medical student at the Medical School of Universidade Federal de Minas Gerais (UFMG).

Mail: larissarezendeg[at]gmail.com

Supervisors

Dr. Rodrigo Gomes da Silva, associate professor of the Department of Surgery at the Medical School of UFMG, coloproctologist and coordinator of the Medical Residency in Coloproctology at Alfa Institute of Gastroenterology of Hospital das Clínicas (HC)-UFMG.

Mail: rodrigogsilva[arroba]uol.com.br

Dr. Ana Carolina Guimarães de Castro, clinical oncologist at Grupo Oncoclínicas. Master and PhD in Gastroenterology by UFMG.

Mail: aguimaraescasto[arroba]uol.com.br

Dr. Júlio Guerra Domingues, radiologist and professor of the Department of Anatomy and Image at the Medical School of UFMG.

Mail: jgdjulio[at]gmail.com.br

Reviewers

Almir Marquiore, Bruno Chaves, Gabriella Shiomatsu, Leandra Diniz and Rafael Arantes.

Translated by

Melina Assunção Gomes de Araújo, 5th year medical student at the Medical School of UFMG.

Mail: melinaraujoo[at]gmail.com

Test question

(UNIFESP 2015 - Intensive Care Medicine) A 49-years-old patient presented to the emergency room with his brother, who reported that he had a sudden loss of strength in the left arm, he also presented a difficulty of communication. Both symptoms started 35 minutes ago during physical activity at the gym. Past medical history: systemic arterial hypertension and smoking. On examination: alert, obeying commands, total right hemiparesis with brachio-facial predominance (muscle strength grade II). Afasia, no stiff neck, isochoric and photoreactive pupils. Blood pressure: 190/110 mmHg. Heart rate: 88bpm; RF: 14. Computed tomography of the brain and laboratory tests were normal. What is the probable diagnosis and the more appropriate management?

a) Subarachnoid hemorrhage. The evolution should be carefully observed, with magnetic resonance imaging performed as soon as possible.

25%

b) Transient ischemic stroke. In view of the time lapse, it is not a candidate for reperfusion thrombolytic therapy. It is not recommended to control the hypertension in this acute phase.

25%

c) Ischemic stroke. In view of the time lapse, he is not a candidate for reperfusion thrombolytic therapy. Aspirin and neurological surveillance are recommended.

25%

d) Ischemic stroke. In view of the time lapse, this is a candidate for reperfusion thrombolytic therapy with r-tPA. However, it is necessary an adequate pressure control and to make sure of the absence of other contraindications.

25%

e) Hemorrhagic stroke. It is recommended an adequate pressure control and repeat the tomography in the first 24 hours.

25%
   

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